
==== Front
Plast Reconstr Surg Glob Open
Plast Reconstr Surg Glob Open
GOX
Plastic and Reconstructive Surgery Global Open
2169-7574
Lippincott Williams & Wilkins Hagerstown, MD

GOX-D-23-01107
00026
10.1097/GOX.0000000000006122
3
Craniofacial/Pediatric
Original Article
Pediatric Desmoid Tumor of the Head and Neck: A Systematic Review and Modified Framework for Management by Age Group
Torres Bryan S. MS *
Brown Hannah G. BS †
Nuñez Julisa MS ‡
Abongwa Chenue MD §
Hajjar Fouad M. MD §
Sawh-Martinez Rajendra F. MD, MHS ¶
Lopez Joseph MD, MBA, FAAP ¶∥
From the * School of Medicine, Tulane University, New Orleans, La.
† School of Medicine, University of Central Florida, Orlando, Fla.
‡ School of Medicine, Georgetown University, Washington, D.C.
§ Division of Medical Oncology, AdventHealth for Children, Orlando, Fla.
¶ Division of Pediatric Plastic and Reconstructive Surgery, Department of Pediatric Surgery, AdventHealth for Children, Orlando, Fla.
∥ Division of Pediatric Head and Neck Surgery, Department of Pediatric Surgery, AdventHealth for Children, Orlando, Fla.
Joseph Lopez, MD, MBA, FAAP, Division of Pediatric Head and Neck Surgery, Department of Pediatric Surgery, AdventHealth for Children, Orlando, FL, E-mail: joseph.lopez.md@adventhealth.com, Instagram: @drjosephlopez, Twitter: @drjosephlopez
9 2024
09 9 2024
12 9 e61225 12 2023
1 7 2024
Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

Background:

Unlike in adults, pediatric head and neck desmoid tumors (DTs) have greater capacity to interfere with normal anatomical development. Therefore, adequate interventions and management must be defined. We aimed to provide the most comprehensive systematic review on pediatric head and neck DTs to date, including assessment of lesion location predominance, intervention, and management, and examination of any associations between age and outcomes like surgical margin status, recurrence, and complications.

Methods:

A systematic literature review was conducted between January 1990 and December 2023 using PubMed, Scopus, and MEDLINE databases following the Preferred Reporting Items for Systematic Review and Meta-Analyses 2020 guidelines. We aimed to elucidate intervention and management strategies by studying various outcomes in 0–11 and 12–21 year olds.

Results:

The literature search yielded 44 studies, totaling 121 patients. Most head and neck DTs localized to the mandible, cranium, and neck; occurred early (P = 4.18 years); and underwent local resection with positive margins. Older and younger patients shared no difference in complication or recurrence rates.

Conclusions:

We found recurrence is likely to occur with positive margins. Because standard treatment of DTs is surgical resection with negative margins, if technically feasible with reasonable associated morbidity, we suggest additional resection of the tumor to achieve negative margins. We also recommend more robust follow-up data collection, not only due to desmoid’s high recurrence and data paucity in patients older than 12 years, but also, to better establish best management practices.

OPEN-ACCESSTRUE
COUNTRYUNITED STATES
SDCT
==== Body
pmcTakeaways

Question: We aimed to perform the most comprehensive review on pediatric head and neck desmoid tumors (DTs), focusing on location, intervention, and management, while exploring the relationship between surgical margins, recurrence, and complications.

Findings: Pediatric head and neck DTs primarily affect the mandible, neck, and cranium (73%). We found that positive surgical margins were more common than negative ones, but margin status did not influence recurrence rates. Surgical resection remains the first-line treatment, though recurrence is common, necessitating long-term follow-up.

Meaning: Early and thorough surgical intervention is crucial, but given the high recurrence rates, careful long-term monitoring is essential for managing pediatric head and neck DTs.

INTRODUCTION

Desmoid tumors (DTs), also known as desmoid-type fibromatosis or aggressive fibromatosis, are rapidly growing, benign, soft-tissue tumors that arise from mesenchymal tissues and fibroblastic cells.1 These tumors have the ability to develop quickly and without distant metastasis, allowing mass growth and infiltration of local structures.2 DTs are known for local recurrence after resection and have the capability to cause major morbidity, earning a classification as an intermediate/locally aggressive tumor by the World Health Organization.3 This tumor type originates from both germline variations and somatic mutations that include adenomatous polyposis coli or β-catenin, respectively.4 DTs are most commonly found within the abdominal cavity or wall (60%) but extraabdominal (40%) locations have also been described in the literature.2,5,6 In the head and neck region, the occurrence is noted as 12%–15%.2 Although head and neck DTs make up a smaller percentage, it is important to understand their unique characteristics and treat them accordingly.6 In the pediatric population, the rapid uncontrolled growth and nature of DTs can easily interfere with complex head and neck anatomy and endocrine development, and can present unique challenges with intervention surgically and therapeutically (Fig. 1).6 Given the complex nature and presentation of DTs with a lower prevalence in the head and region and within the pediatric population, intervention and management must be strategically planned and executed. This study aimed to provide the most comprehensive systematic review to date on head and neck DT occurrence in the pediatric population, focusing on patient age, intervention, and outcomes. In addition, we further analyzed the literature on surgical margin status, recurrence, need for reconstruction, and complications by age.

Fig. 1. DT in the neck of a pediatric patient before resection. Intraoperative image of desmoid in the neck of a pediatric patient after lateral retraction of the sternocleidomastoid muscle and before resection. After total resection, the mass measured 6.2 × 6.0 × 4.0 cm.

METHODS

Literature Search

A systematic review was conducted following the Preferred Reporting Items for Systematic Review and Meta-Analyses 2020 guidelines, using PubMed and cross-referencing of MEDLINE and Scopus database to identify citations related to DTs of the head and neck within the pediatric population in the available English-language literature between January 1990 and December 2023. The timeframe would provide us with the most recent and updated literature. To ensure that we captured DTs, the literature search was conducted using the following terms (and their combinations): “fibromatosis, aggressive/etiology”; at least one of the following terms: “fibromatosis, aggressive/therapy” OR “fibromatosis, aggressive/complications” OR “fibromatosis, aggressive/diagnostic imaging” OR “fibromatosis, aggressive/drug therapy” OR “fibromatosis, aggressive/mortality” OR “fibromatosis, aggressive/pathology” OR “fibromatosis, aggressive/physiopathology” OR “fibromatosis, aggressive/prevention” and control” OR “fibromatosis, aggressive/surgery.” Studies were initially screened for relevance based on their titles to include only the DT pediatric population (less than 21 years of age as defined by the American Academy of Pediatrics).3 Reviews, conference proceedings, nonhead and neck studies, and duplicates were excluded. Articles were included only if they contained their own cases and the authors discussed findings within the context of their own case. The articles of retrieved studies were further screened for relevant articles. Full-text review of the remaining articles was performed. Articles were categorized by the level of evidence described by the American Society of Plastic Surgeons levels of evidence scale.

Quality Assessment

The methodological quality of articles included in this systematic review was accessed by using the Cochrane Collaboration tool validity questionnaire for assessing risk of bias.7

Data Extraction

Data were collected by multiple authors (B.S.T., H.G.B., J.N.) using a data abstraction form. Extracted variables included study design, study period, number of patients, patient demographics (eg, age, sex, and racial ethnicity, if available), pathology, tumor location, anamnesis duration, intervention, reconstruction, recurrence, follow-up, and perioperative complications. Articles with tumor locations not exclusive to the head and neck, data on patients older than 21 years (or with a median age group greater than 21), or incomplete patient information were excluded. Patients included in the analysis were stratified into a young (age: 0–11 y) and older (age: 12–21 y) group for comparison purposes. This categorization is common in studies to group patients into prepubescent and postpubescent groups.8–10 Tumor location was categorized into groups defined by the anatomic area of predominance, as follows: mandibular (mandible and submandibular, masseter muscle, masticator space, infratemporal fossa, pterygopalatine); maxillary (maxilla, buccal); nasal (nasal, paranasal); neck (sternocleidomastoid, parapharyngeal, clavicular fossa); cranial (skull based, cranium); oral (oropharynx, tongue); orbital (sino-orbital, orbital, intraconal, medial canthus, nasolacrimal); sinus (maxillary sinus, frontal sinus, ethmoid sinus/air cells); pharyngeal (pharynx, hypopharynx); laryngeal (larynx, epiglottis); salivary (parotid); and other (nonspecified in the head and neck, subscapular region, shoulder, external ear). Tumors that were in more than one location were categorized into two location groups. We performed a pooled analysis to acquire margin status, recurrence rate, and complication rate. Major complications were defined by adverse events peri- or postoperatively that required surgical intervention. In contrast, minor complications were defined as those that could be managed medically or by a bedside procedure, such as infection treated with antibiotics.

Statistical Analysis

Data distribution and descriptive analysis were performed using Microsoft Excel software. Two-way t-tables and chi-square tests were used to prove or disprove our hypotheses. All chi-square critical values equated to 3.841, which is associated with a degree of freedom of 1 (df = 1) and a 95% confidence interval, or α = 0.05, when drawing conclusions about significance. The hypotheses tested are as follows: (1) that positive surgical margins would yield a greater likelihood of recurrence than cases with negative margins would; (2) that a statistically significant difference would exist between the recurrence rates of younger children, defined as ages 0–11 years, and older children, defined as ages 12–21 years; (3) that a statistically significant difference would exist between the rates of complications in the defined age groups; and (4) that experiencing positive or negative surgical margins may be predicated on age group. Odds ratios were also used to explore noted differences between comparison groups.

RESULTS

Search Results and Study Characteristics

Records identified from database searching (n = 514) were screened for eligibility. After excluding records that did not meet the inclusion criteria (n = 169), a total of 345 titles and abstracts were screened. Of these, 202 records were excluded due to irrelevance to the study, incomplete or lack of specific information such as grouped study populations, or inclusion of data on both pediatric and adult populations upon further review of abstract. Consequently, 143 titles and abstracts were deemed eligible for full-text assessment. After a thorough evaluation of the full-text articles, 99 were excluded from the final analysis. The reasons for exclusion included studies involving adult populations (n = 7), those not specifically focusing on head and neck issues (n = 17), and articles with incomplete or insufficient information (n = 75). Ultimately, 44 studies met the eligibility criteria and were included in the analysis. (Fig. 2). Of these 44 articles, we included one prospective nonrandomized trial (level II), nine retrospective case series (levels III and IV), and 30 case reports (levels V). Most of the studies were published in the United States. (See table, Supplemental Digital Content 1, which shows characteristics of included articles. http://links.lww.com/PRSGO/D470.)

Fig. 2. Preferred reporting items for systematic reviews and meta-analyses flowchart detailing study inclusion.

Patient Characteristics

Of the 121 patients identified in these 44 articles that met inclusion criteria, there were 53 male patients (43%) and 40 female patients (33%). The sex of 28 patients went unreported (23%). Patients were dichotomized into two age groups in years (0–11 or 12–21 years) with a mean of 4.18 years and a range from 0 days to 21 years. Tumor location was categorized into 12 different groups based on the anatomical proximity of structures in which the tumor was predominantly located with certain tumors meeting more than one location group. Tumor location was as follows: 66 patients had mandibular (46%), 21 had neck (15%), 16 had cranial (11%), nine had oral (6%), eight had orbital (5%), four had nasal (3%), four had maxillary (3%), three had sinus (2%), three had pharyngeal (2%), three had salivary (2%), three had other (2%), and one had laryngeal (<1%) (Fig. 3). The mean follow-up for all these patients was 3.46 years. (See table, Supplemental Digital Content 2, which displays how tumors were categorized according to where the tumor was predominantly located. http://links.lww.com/PRSGO/D471.)

Fig. 3. The number of subjects included in the analysis by head and neck location group.

Management and Interventions

Of the 121 patients identified for this study, the treatment and intervention type were collected. The intervention types were categorized as follows: surgical resection, chemotherapy only, radiation only, and a combination of neoadjuvant therapy, adjuvant therapy, radiation followed by resection, resection followed by radiation, resection followed by chemotherapy and radiation, or chemotherapy, resection, and radiation. Of the surgical resection, 77 patients (63%) underwent total surgical resection (defined as >90% tumor removal), three patients had (2.5%) other (curettage), two patients (1.6%) had debulking, two patients (1.6%) had an incomplete resection, and one patient (0.78%) had a biopsy only. Of those receiving chemotherapy, nine patients (7.4%) were treated with methotrexate and vinblastine, and eight patients (6.6%) belonged to “other” (defined as nonmethotrexate/vinblastine). Of the combination treatment group, five patients (4.1%) had neoadjuvant therapy; one patient (0.83%) had adjuvant therapy; one patient (0.83%) had radiation followed by resection; two patients (1.65%) had resection followed by radiation; one patient (0.83%) had resection followed by chemotherapy and radiation; and one patient (0.83%) had chemotherapy, resection, and radiation. Of the patients in age group 0–11 years, 22 patients (18.18%) underwent reconstruction, whereas in the age group 12–21 years, four patients (3.31%) had reconstructive surgery. (See table, Supplemental Digital Content 3, which shows a summary of management and interventions. http://links.lww.com/PRSGO/D472.)

Outcomes

A summary of the outcomes showed that of the 77 patients who underwent total resection, 27 patients (35%) had positive margins, and six patients (7%) had negative margins. These data were recorded only if it was part of the initial resection. The rate of recurrence for age group 0–11 years was 25 patients (20.6%), whereas for those in the age group 12–21, it was four patients (3.31%). Complications for all 121 patients were also recorded and categorized as major or minor. A major complication was defined as any intervention following an adverse event requiring anesthesia, whereas a minor complication was defined as not requiring anesthesia or managed medically at the bedside. Of the 121 patients, seven patients (5.8%) had major complications and 18 patients (14.9%) had minor complications. The remainder of the patients had either no complications or complications were unreported. (See table, Supplemental Digital Content 4, which displays a summary of outcomes and complications reported. ‡Studies with no complications or unreported complications were not collected for the respective age groups, only tabulated for a total. http://links.lww.com/PRSGO/D473.)

Comparison of Outcomes in Older Children versus Younger Children

When investigating recurrence relative to age group, our analysis revealed no statistically significant difference in recurrence in head/neck DTs in young patients (0–11 year range) when compared with older patients (12–21 year range) [odds ratio (OR) 2.4:1; P = 0.154]. When comparing surgical margins achieved in younger and older patients, our pooled chi-squared analysis also revealed no statistically significant difference in positive/negative margins (OR 1.639:1; P = 0.201). When comparing young versus older patients and their rate of complications, our analysis also found no statistically significant difference (OR 1.5:1; P = 0.675). Lastly, when comparing the dependence of recurrence rates on positive surgical margins, our analysis also found no association (OR 1.078:1; P = 0.299). (See table, Supplemental Digital Content 5, which shows a summary of chi-squared analysis by comparison group. No statistical significance is indicated. http://links.lww.com/PRSGO/D474.) All studies reviewed are shown in Supplemental Digital Content 6.1,5,11–53 (See table, Supplemental Digital Content 6, which displays all included articles. http://links.lww.com/PRSGO/D475.)

DISCUSSION

Pediatric DTs have been found to have a high recurrence rate, be locally invasive, and be fast-growing.2,22,53 In the head and neck region of the pediatric patients under review, the mandible, neck, and cranium made up the top three locations (approximately 73%) of the total DTs collected. Thus, prompt diagnosis and intervention may be indicated to reduce the likelihood of more devastating mass effects. This is the most comprehensive systematic review to date on the management of DTs of the head and neck region in the pediatric population. Our review found that age was not a predicting factor for recurrence, achieved surgical margin status, or complications. Although the data showed that children in the age group 0–11 years were 2.4 times more likely to experience recurrence than those in the 12- to 21-year-old age group, our statistical analysis did not achieve statistical significance, likely due to low statistical power.

Our review found that age was not a predicting factor for recurrence, achieved surgical margin status, or complications. Although the data showed that children in the age group 0–11 years were 2.4 times more likely to experience recurrence than those in the 12- to 21-year-old age group, our statistical analysis did not achieve statistical significance due to low statistical power.

We found that approximately 64% of patients underwent a total surgical resection, which was also the most common modality of treatment regardless of margin status. Although our review found that 17 patients underwent chemotherapy treatment as the main modality of treatment, only two patients became tumor free. A total of five patients experienced recurrences (29.4%) and 10 patients were left with stable disease (58.8%). A total of five patients underwent brachytherapy treatment only. Unfortunately, three of the five of whom follow-up data were provided experienced recurrences (100%). In comparison, patients utilizing surgical excision only as the main form of treatment experienced a recurrence of 29.9% (23 of 77) and a lower stable tumor rate of 2.6% (two of 77), supporting surgical intervention as the first-line treatment for pediatric head and neck.1,2,17,22,44,51–53

Of the total population of 121 patients reviewed, only 48 cases reported margins. Of those, 40 had positive margins (83%) while eight had negative margins (17%). Despite surgical margins going unreported in numerous studies, the outcome of those reported suggests the difficulty of complete DT resection. Contrary to our expectations from previous suggestions made in the literature,1,17,22,44,51–53 our sample yielded no evidence that recurrence rates were greater in those with positive margins than those without. Future analyses with larger sample sizes are necessary to examine this more thoroughly. Whether using vincristine and/or vinblastine as pretreatment or postoperatively following positive surgical margins, our study results showed limited efficacy for the role of chemotherapeutic agents in treating head and neck DTs.14,17,24,27,38,44,50 Introduction of cytotoxic drugs that have shown a lack of clear benefit in this pediatric population while inciting adverse effects such as peripheral neuropathy and DNA toxicity prompt the need for alternative management.46 Therefore, and similarly to patients with negative margin status, our findings support that patient lesions requiring positive surgical margins warrant close, long-term follow-up and observation. If recurrences arise, then attempts to reexcise the lesion may be considered. Finally, although early trials of brachytherapy as monotherapy also produced increased rates of recurrence, its postoperative use is yet undetermined.11

When analyzing complication status, we found that those with stable disease and tumor-free status experienced a lower percentage of complications (both at 20%) compared with those experiencing recurrences (46%). This finding may be due to nature of an invasive surgical intervention in comparison with interventions causing less complications due to being surgically conservative. Our analysis supported neither the correlation between surgical margins and age group nor that a significant difference exists between younger and older pediatric patient complication rates. It must be noted that these results are not generalizable for those aged 12–21 years, as the sample size of this group only totaled 10 patients (total of ages 0–11, n = 79). Of those 10 patients, only eight cases reported whether complications occurred (in the 0–11 age group, 46 reported absence or presence of complications).

To date, this is the only systematic review evaluating management and outcomes in the pediatric head and neck DT population. Our study has several limitations that warrant consideration. Confounding variables introduced limitations within our statistical findings. For example, surgical interventions requiring reconstruction may have influenced the likelihood of experiencing major complications because of additional intervention. In addition, the patient population reviewed was positively skewed with a mean age population of 4.18. Furthermore, all articles included in our analysis were case reports, case series, or retrospective studies with low levels of evidence. Future studies of higher-level evidence must be conducted with more representation of all pediatric ages to conclude significant differences. A multicenter study with a greater population size may be effective. A multivariate analysis will be required to map true correlations between the many existing independent variables found within this study.

CONCLUSIONS

Although relatively uncommon, pediatric head and neck DTs require special attention due to their aggressive nature in a complex area with vital structures. Outlining that 73% of pediatric head and neck DTs are in the mandible, neck, and cranium gives surgeons an understanding of where they are most likely to come across these lesions, benefitting their surgical preparation and planning. Our review also suggests that of those reporting surgical margins, positive margins were achieved more often than negative surgical margins, emphasizing the difficulty of excising these lesions completely. Despite finding that surgical margin status had no impact on recurrence rates, and that all outcomes evaluated were age-independent, the mean age of occurrence for these tumors is 4.18 years, which is very young. Given that the standard treatment of DTs is surgical resection with negative margins, if technically feasible with reasonable associated morbidity, we suggest additional resection of the tumor to achieve negative margins. If not feasible, due to high morbidities such as damage to vital structures and unfavorable scarring, then we advise a more conservative surgical approach, and to continue educating patients and their families that these lesions have a one in five chance of recurrence. Finally, given this high postoperative recurrence rate regardless of margin status, long-term follow-up and postoperative data collection is a critical part of proper management of all patients with pediatric head and neck desmoid and is necessary for the evolving determination of patient-centered best practices.

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article.

Supplementary Material

Published online 9 September 2024.

Disclosure statements are at the end of this article, following the correspondence information.

Related Digital Media are available in the full-text version of the article on www.PRSGlobalOpen.com.

Torres and Brown contributed equally to this work.
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REFERENCES

1. Sinno H Zadeh T . Desmoid tumors of the pediatric mandible: case report and review. Ann Plast Surg. 2009;62 :213–219.19158537
2. Martínez Trufero J Pajares Bernad I Torres Ramón I . Desmoid-type fibromatosis: who, when, and how to treat. Curr Treat Options Oncol. 2017;18 :29.28439797
3. Kumar SS Rajeevan K Devarajan E . Desmoid-type fibromatosis-clinical study of an uncommon disease. Indian J Surg Oncol. 2020;11 :71–74.32205974
4. Le Guellec S Soubeyran I Rochaix P . CTNNB1 mutation analysis is a useful tool for the diagnosis of desmoid tumors: a study of 260 desmoid tumors and 191 potential morphologic mimics. Mod Pathol. 2012;25 :1551–1558.22766794
5. Zhao CX Dombrowski ND Perez-Atayde AR . Desmoid tumors of the head and neck in the pediatric population: has anything changed? Int J Pediatr Otorhinolaryngol. 2021;140 :110511.33271436
6. Rhim JH Kim JH Moon KC . Desmoid-type fibromatosis in the head and neck: CT and MR imaging characteristics. Neuroradiology. 2013;55 :351–359.23338838
7. Higgins JPT Thomas J Chandler J (Eds.). Cochrane Handbook for Systematic Reviews of Interventions. Version 6.4. Updated August 2023. Cochrane; 2023. Available at www.training.cochrane.org/handbook. Accessed August 11, 2024.
8. Lin Z Yang R Li K . Establishment of age group classification for risk stratification in glioma patients. BMC Neurol. 2020;20 :310.32819307
9. Kapelari K Kirchlechner C Högler W . Pediatric reference intervals for thyroid hormone levels from birth to adulthood: a retrospective study. BMC Endocr Disord. 2008;8 :15.19036169
10. Job KM Gamalo M Ward RM . Pediatric age groups and approach to studies. Ther Innov Regul Sci. 2019;53 :584–589.31311309
11. Zhong YW Lyu XM Shi Y . Long-term result of 125 I seed brachytherapy for pediatric desmoid tumor in the head and neck. Pediatr Blood Cancer. 2023;70 :e30037.36184787
12. Downie EM Amend CE Miranda A . Treatment of orbital desmoid-type fibromatosis with sorafenib. Ophthalmic Plast Reconstr Surg. 2022;38 :e144–e147.35470290
13. Albokashy MS Halawani MS Eshky AT . Massive congenital cervicofacial desmoid-type fibromatosis in a 5-month-old infant. J Surg Case Rep. 2021;2021 :rjab206.34055293
14. Curry DE Al-Sayed AA Trites J . Oral losartan after limited mandibulectomy for treatment of desmoid-type fibromatosis. Ear Nose Throat J. 2023;102 :NP49–NP52.33491484
15. Peroša N Urbančič J Felbabić T . Desmoid-type fibromatosis of paranasal sinuses with intracranial extension in a child—a case-based review. Childs Nerv Syst. 2021;37 :3673–3680.34611763
16. Daram SP Timmons C Mitchell RB . Desmoid fibromatosis of the maxilla. Ear Nose Throat J. 2020;99 :NP6–NP8.31937133
17. Paul A Blouin MJ Minard-Colin V . Desmoid-type fibromatosis of the head and neck in children: a changing situation. Int J Pediatr Otorhinolaryngol. 2019;123 :33–37.31059930
18. Khaladj-Ghom A Isaiah A Caccamese JF Jr . Facial desmoid tumor in a 2-year-old. Ear Nose Throat J. 2020;99 :NP3–NP5.31536374
19. Chang B Ha JF Zopf D . Treatment of extensive post tonsillectomy oropharyngeal stenosis secondary to fibromatosis. Int J Pediatr Otorhinolaryngol. 2018;107 :107–109.29501289
20. Lee JW Bewley AF Senders CW . Marginal versus segmental mandibulectomy for pediatric desmoid fibromatosis of the mandible—two case reports and review of the literature. Int J Pediatr Otorhinolaryngol. 2018;109 :21–26.29728178
21. Moro A De Angelis P Gasparini G . Orbital desmoid-type fibromatosis: a case report and literature review. Case Rep Oncol Med. 2018;2018 :1684763.29707396
22. Risoud M Mortuaire G Leroy X . Desmoid tumours of the head and neck in children: review of management. Eur Ann Otorhinolaryngol Head Neck Dis. 2017;134 :155–160.27988199
23. Nair KK Chaudhuri K Lingappa A . Aggressive fibromatosis of the oral cavity in a 5 year old boy: a rare case report. Pan Afr Med J. 2017;27 :47.28819469
24. Xia C Zhu Q Yue C . Sonography used in the infantile desmoid fibromatosis of postcricoid area: a case report. Medicine (Baltim). 2017;96 :e8500.
25. Clark CM Bann DV Zacharia TT . Unilateral neck swelling in a pediatric patient. JAMA Otolaryngol Head Neck Surg. 2017;143 :423–424.28033435
26. Li L Jensen JN Szabo S . Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report. J Neurosurg Pediatr. 2016;25 :703–707.27635978
27. Zheng Z Jordan AC Hackett AM . Pediatric desmoid fibromatosis of the parapharyngeal space: a case report and review of literature. Am J Otolaryngol. 2016;37 :372–375.27040413
28. Miyashita H Asoda S Soma T . Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature. J Med Case Rep. 2016;10 :173.27286970
29. Peña S Brickman T StHilaire H . Aggressive fibromatosis of the head and neck in the pediatric population. Int J Pediatr Otorhinolaryngol. 2014;78 :1–4.24290952
30. Burlini D Conti G Bardellini E . Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management. Eur J Paediatr Dent. 2013;14 :333–334.24313589
31. Bede SY Ismael WK Abdullah BH . Submandibular juvenile fibromatosis. J Craniofac Surg. 2013;24 :e411–e413.23851885
32. Zhou Y Zhang Z Fu H . Clinical management of pediatric aggressive fibromatosis involving the mandible. Pediatr Blood Cancer. 2012;59 :648–651.22556010
33. Wilks DJ Mowatt DJ Merchant W . Facial paediatric desmoid fibromatosis: a case series, literature review and management algorithm. J Plast Reconstr Aesthet Surg. 2012;65 :564–571.22154716
34. Lu D Yang H Chen F . Aggressive fibromatosis (desmoid tumour) of the pharynx. ANZ J Surg. 2011;81 :734–736.22295317
35. Friedrich RE Grzyska U Scheuer HA . Desmoid-type infantile fibromatosis of the mandible: case report with long-term follow-up. In Vivo. 2010;24 :877–881.21164048
36. Sharma A Ngan BY Sándor GK . Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review. J Pediatr Surg. 2008;43 :1596–1604.18778992
37. Lakhan SE Eager RM Harle L . Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review. J Hematol Oncol. 2008;1 :3.18577255
38. Hartstein ME Thomas SM Ellis LS . Orbital desmoid tumor in a pediatric patient. Ophthalmic Plast Reconstr Surg. 2006;22 :139–141.16550064
39. De Riu G Meloni SM Raho MT . Complications of mandibular reconstruction in childhood: report of a case of juvenile aggressive fibromatosis. J Craniomaxillofac Surg. 2006;34 :168–172.16549363
40. Watzinger F Turhani D Wutzl A . Aggressive fibromatosis of the mandible: a case report. Int J Oral Maxillofac Surg. 2005;34 :211–213.15695054
41. Styczynski J Lasek W Wysocki M . Calcified fibromatosis of the neck in 4-year old girl: rapid growth, rapid therapy. Int J Pediatr Otorhinolaryngol. 2005;69 :847–852.15885340
42. Buitendijk S van de Ven CP Dumans TG . Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 2005;104 :1090–1099.16015632
43. Seper L Bürger H Vormoor J . Agressive fibromatosis involving the mandible--case report and review of the literature [published correction appears in Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Feb;99(2):254]. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99 :30–38.15599346
44. Lackner H Urban C Benesch M . Multimodal treatment of children with unresectable or recurrent desmoid tumors: an 11-year longitudinal observational study. J Pediatr Hematol Oncol. 2004;26 :518–522.15284591
45. Roychoudhury A Parkash H Kumar S . Infantile desmoid fibromatosis of the submandibular region. J Oral Maxillofac Surg. 2002;60 :1198–1202.12378500
46. Tostevin PM Wyatt M Hosni A . Six cases of fibromatosis of the head and neck in children. Int J Pediatr Otorhinolaryngol. 2000;53 :235–244.10930641
47. Sato K Kawana M Nonomura N . Desmoid-type infantile fibromatosis in the mandible: a case report. Am J Otolaryngol. 2000;21 :207–212.10834557
48. Perez-Cruet MJ Burke JM Weber R . Aggressive fibromatosis involving the cranial base in children. Neurosurgery. 1998;43 :1096–1102.9802853
49. De Santis D . Fibromatosis of the mandible: case report and review of previous publications. Br J Oral Maxillofac Surg. 1998;36 :384–388.9831061
50. Martínez-Lage JF Acosta J Sola J . Congenital desmoid tumor of the scalp: a histologically benign lesion with aggressive clinical behavior. Childs Nerv Syst. 1996;12 :409–412.8869779
51. Hoffman CD Levant BA Hall RK . Aggressive infantile fibromatosis: report of a case undergoing spontaneous regression. J Oral Maxillofac Surg. 1993;51 :1043–1047.8355096
52. Carr RJ Zaki GA Leader MB . Infantile fibromatosis with involvement of the mandible. Br J Oral Maxillofac Surg. 1992;30 :257–262.1510902
53. Gronchi A Jones RL . Treatment of desmoid tumors in 2019. JAMA Oncol. 2019;5 :567–568.30703188
